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Benign cephalic histiocytosis: a case report and review
Debra M Jih1, Stephen L Salcedo, Christine Jaworsky
1Department of Dermatology, Metrohealth Medical Center, Case Western Reserve School of Medicine, Cleveland, Ohio 44109-1998, USA.
Insights
Benign cephalic histiocytosis is a rare skin condition in children. This self-healing disorder presents as facial papules and macules, typically resolving by early childhood.
Area of Science:
- Dermatology
- Pediatric Pathology
Background:
- Benign cephalic histiocytosis (BCH) is a rare, non-Langerhans cell histiocytosis.
- It is characterized by spontaneous resolution of skin lesions.
Observation:
- A 9-month-old boy presented with facial papules and macules.
- Lesions spread to the lower extremities and began to regress by 30 months of age.
Findings:
- Histopathology revealed dermal histiocyte proliferation.
- Distinctive ultrastructural findings included comma-shaped bodies and coated vesicles, with an absence of Birbeck granules.
Implications:
- This case highlights the typical presentation and self-limiting course of BCH.
- Understanding its unique histological features aids in differentiating it from other histiocytic disorders.
Abstract:
Benign cephalic histiocytosis is a rare non-Langerhans histiocytosis characterized by a self-healing eruption of papules and macules on the head and neck that occurs during infancy or childhood. Histologic and ultrastructural evaluations show a dermal proliferation of histiocytes with intracytoplasmic comma-shaped bodies, coated vesicles, and desmosome-like structures with an absence of Birbeck granules. We report a case of benign cephalic histiocytosis in a 9-month-old boy who presented with tan papules on his face that spread to his lower extremity and subsequently began to regress at 30 months of age. We review the features of this rare entity through a literature review and discuss the differential diagnosis.